Case 14-1998

Reilly, John J.; Mark, Eugene J.

doi:10.1056/nejm199804303381809

Cited by 12 publications

(1 citation statement)

References 25 publications

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“… 34 – 36 A number of patients have features of both diseases, raising the question of whether they are within the spectrum of one disease or are two distinct clinical disorders that may share an autoimmune pathogenesis with some overlapping symptoms. 37 Kishor et al 38 reviewed 17 patients with sarcoidosis and PBC and suggested that a common pathway contributes to granuloma formation in both disorders. The prevalence of GLD related to PBC is not known, but it seems to be rare based upon case reports and case series.…”

Section: Granulomatous Lung Disease (Gld)mentioning

confidence: 99%

Pulmonary Manifestations among Patients with Primary Biliary Cirrhosis

Köksal

Gürakar

2016

JCTH

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Primary biliary cirrhosis (PBC) is a chronic progressive cholestatic liver disease caused by diffuse inflammation, destruction and fibrosis of the intrahepatic bile ducts, ultimately leading to cirrhosis, portal hypertension and liver failure. The pathogenesis of PBC is incompletely understood, but current data suggest roles for genetic susceptibility and environmental factors. PBC is often thought of as an organ-specific autoimmune disease, which mainly targets the liver; however, lung tissue is also a site for autoimmune involvement of PBC. The pulmonary manifestations of PBC include abnormalities in gas transfer and pulmonary function, subclinical alveolitis, interstitial lung disease, granulomatous lung disease, airway disease, pulmonary hypertension, pulmonary hemorrhage and pleural effusion.

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